For 3 weeks, a previously healthy 24-year-old man had repeated episodes of nonbloody, nonbilious emesis. He denied dysphagia, but he did report a 40-lb (18-kg) weight loss and heartburn. Physical examination findings were normal and a complete blood cell count and metabolic profile were unremarkable.
Esophagogastroduodenoscopy revealed a mildly dilated lower third of the esophagus (A). CT demonstrated a dilated esophagus (B). A barium swallow showed the classic “bird’s beak” appearance of the distal esophagus with proximal esophageal dilatation (C and D). A diagnosis of achalasia was made.Achalasia, although rare, is a well-understood esophageal motility disorder. Its incidence is approximately 0.5 cases per 100,000 per year.1 Achalasia is characterized by degeneration of the myenteric plexus that supplies the esophageal sphincter, with resultant imbalance between excitatory and inhibitory neurons.2 Unopposed cholinergic stimulation leads to increased lower esophageal sphincter (LES) pressure.3 Dysphagia with solids and liquids is the most common initial symptom.2,4 Weight loss typically occurs late in the disease and usually does not exceed 11 to 22 lb (5 to 10 kg).2 Other symptoms include heartburn, regurgitation, difficulty with belching, and chest pain.2
The primary screening test for achalasia is barium esophagography. Manometric findings of elevated resting LES pressure, incomplete LES relaxation, and aperistalsis confirm the diagnosis.2 Because manometry is not readily available, however, the diagnosis is often confirmed by clinical presentation and suggestive radiographic and endoscopic findings. Optimal management should include manometry if possible, especially before therapy is initiated.
This patient underwent laparoscopic Heller myotomy on hospital day 5 and had marked improvement in his symptoms. He is recovering from the profound weight loss.
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8. Kilic A, Schuchert MJ, Pennathur A, et al. Long-term outcomes of laparoscopic Heller myotomy for achalasia. Surgery. 2009;146:826-831.